Türer Özlem Boybeyi, Demir Numan, Ciecieraga Thomas, Günaydın Rıza Önder, Soyer Tutku
Departments of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara.
Department Physiotherapy and Rehabilitation, Hacettepe University Faculty of Health Sciences, Ankara.
Turk J Pediatr. 2019;61(5):804-809. doi: 10.24953/turkjped.2019.05.025.
Boybeyi Türer Ö, Demir N, Ciecieraga T, Günaydın RÖ, Soyer T. Assessment of pediatric cricopharyngeal achalasia with high resolution manometry. Turk J Pediatr 2019; 61: 804-809. Cricopharyngeal achalasia (CPA) is an uncommon cause of oropharyngeal dysphagia (OPD) which is the failure of upper esophageal sphincter (UES) to relax during bolus passage. The diagnostic challenges in OPD have been overcome with the use of high resolution manometry (HRM) in children where a catheter based biomechanical evaluation testing of the oropharyngeal swallowing is performed. Herein, we present a case with severe dysphagia diagnosed as CPA utilizing HRM testing. An 8-year-old boy was seen in our clinic with a two-year history of difficult swallowing, recurrent respiratory tract infections, hypoxia and seizure secondary to aspiration. Esophagography revealed an indentation of the cricopharangeal muscle (CPM) in the cervical part of the esophagus. Videofluroscopic swallow studies (VFSS) revealed cricopharyngeal bar at level of C5-6 and diffuse dysmotility in esophagus. Conventional esophageal manometry revealed absence of peristaltic activity throughout the esophagus. Esophagogastroduodenoscopy revealed narrowing in upper esophagus that with applied force allowed passage of the endoscope. The patient underwent UES dilatation 6 times. He had temporary relief of symptoms. Since he did not have sustained response to dilatation, a botulinium toxin (5IU/each quadrant) injection (BTI) to CPM was performed twice. His symptoms recurred at the end of 3 months. HRM was performed revealing weakness of CPM and uncoordinated contractions and relaxations in UES. We could not reliably differentiate if HRM findings were a sign of primary illness or secondary to BTI. Swallowing rehabilitation was adjusted based on HRM findings. He is still under follow-up with mild dysphagia to certain solids. CPA is an uncommon cause of dysphagia with limited therapeutic options. HRM should be performed in OPD before considering any treatment modality including esophageal dilatation and BTI. HRM has become gold standard diagnostic tool in OPD that provides objective evaluation of pharyngeal and UES motility in children.
博伊贝伊·蒂勒尔·Ö、德米尔·N、切西埃拉加·T、居纳伊丁·RÖ、索耶·T。高分辨率测压法评估小儿环咽肌失弛缓症。《土耳其儿科学杂志》2019年；61: 804 - 809。环咽肌失弛缓症（CPA）是口咽吞咽困难（OPD）的一种罕见病因，口咽吞咽困难是指食团通过时食管上括约肌（UES）不能松弛。小儿OPD的诊断难题已通过高分辨率测压法（HRM）得以克服，HRM是一种基于导管的对口咽吞咽进行生物力学评估的检测方法。在此，我们报告一例利用HRM检测诊断为CPA的严重吞咽困难病例。一名8岁男孩因吞咽困难、反复呼吸道感染、缺氧以及因误吸继发癫痫发作两年前来我院就诊。食管造影显示食管颈部环咽肌（CPM）有压痕。视频荧光吞咽造影研究（VFSS）显示C5 - 6水平有环咽肌条带，食管存在弥漫性运动障碍。传统食管测压显示整个食管无蠕动活动。食管胃十二指肠镜检查显示食管上段狭窄，用力后内镜可通过。该患者接受了6次UES扩张治疗。症状有短暂缓解。由于对扩张治疗无持续反应，遂对CPM进行了两次肉毒杆菌毒素（每象限5IU）注射（BTI）。3个月后症状复发。HRM检查显示CPM功能减弱，UES收缩和舒张不协调。我们无法可靠地区分HRM结果是原发性疾病的表现还是BTI的继发结果。根据HRM结果调整了吞咽康复治疗。他仍在随访中，对某些固体食物有轻度吞咽困难。CPA是吞咽困难的罕见病因，治疗选择有限。在考虑包括食管扩张和BTI在内的任何治疗方式之前，应对OPD患者进行HRM检查。HRM已成为OPD的金标准诊断工具，可对儿童咽部和UES运动进行客观评估。
